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F Mateos-Beato

Publications and source records attributed to F Mateos-Beato.

11 recordsLinked to original sources
PubMed2000

Generalized spike-and-wave patterns in children: clinical correlates.

All electroencephalograms performed in our institution between 1980 and 1990 were reviewed. The clinical characteristics of children with epilepsy and generalized spike-and-wave (SW) patterns were analyzed. The SW patterns were classified according to their frequency. Electroencephalograms of 154 children with epilepsy revealed SW patterns. Absence seizures were the most common first seizure, but partial seizures were frequent. More than 40% had several types of seizures. Sixty percent of the epileptic syndromes were generalized, but almost 25% were partial. The typical SW pattern was associated with absence seizures, a normal examination and computed tomographic scan, idiopathic generalized epilepsies, monotherapy, freedom from seizures, and lack of recurrence. The slow SW pattern was associated with West syndrome; a younger age at seizure onset; atonic, myoclonic, tonic, and partial simple seizures; an abnormal examination and computed tomographic scan; cryptogenic or symptomatic generalized epilepsy or symptomatic partial epilepsy; polytherapy; and poor seizure control. The fast SW pattern was associated with secondary generalized, partial, tonic-clonic, and complex partial seizures; a normal computed tomographic scan; cryptogenic partial epilepsy; isolated seizures; and seizure recurrence. Epilepsy with a typical SW pattern should be considered benign, epilepsy with a slow SW pattern malignant, and epilepsy with a fast SW pattern treacherous.

Adolescent↗
PubMed1998

[The prognosis of epilepsy in children with generalized spikes and wave disc charges in electroencephalographic recording].

INTRODUCTION: Epilepsy is a chronic disorder. The prognosis of one particular subtype, that of children with generalized spike and wave discharges (P-O) on the electroencephalogram (EEG), is unknown. OBJECTIVE: To determine the prognosis of children with epilepsy with P-O on EEG and the factors which affect this. PATIENTS AND METHODS: All EEGs done over ten years (39,322) were reviewed, and the epileptic patients under 14 years old identified. Absence and recurrence of crises were evaluated and the factors influencing them analyzed. Minimum significance was p < 0.05. The chi square test, Student's t test and Kaplan-Meier method were used. Logistic regression models were devised. RESULTS: Of the children studied, 78% became crisis-free. After monovariant and multivariant analysis the only conditions which fitted the regression models were: abnormal pregnancy; West syndrome or learning difficulties; having certain types of crises, myoclonic, clonic-tonic, partial simple or complex crises; a normal EEG with hypsarrhythmia, slow or fast P-O and requiring multiple medication. When medication ceased, 31.4% had recurrence of the episodes. On monovariant and multivariant analysis, protective factors were absence of attacks and risk factors were a family history of epilepsy or an EEG with rapid P-O. CONCLUSIONS: The overall prognosis for epileptic children with P-O is good. There are factors which improve or worsen the pronosis, but these cannot be modified.

Child↗
PubMedDate not supplied

[Epilepsy and CNS malformations].

INTRODUCTION: Malformations of cortical development are currently considered to be one of the commonest causes of mental retardation and epilepsy. DEVELOPMENT: New neuroimaging techniques have helped diagnosis of these conditions during life and the recognition of new anatomo-clinical syndromes. Although the true incidence of these lesions as a cause of epilepsy is unknown, data from surgical patients indicate that this is the commonest pathology found in children operated on for intractable crises. Most of these patients have focal or generalized crises associated with mental retardation, with a wide range or severity. CONCLUSION: In this review we analyze the clinical features, electroencephalographic findings and the prognosis of epilepsy secondary to these malformations [REV NEUROL 1999; 28: 136-40].

Adolescent↗
PubMedDate not supplied

[Arteriovenous malformation in neurofibromatosis type 1. A case report and review of the literature].

INTRODUCTION: Neurofibromatosis type I may be accompanied by cerebrovascular complications, mainly stenosis or aneurysms and more rarely vertebral arteriovenous fistulas and malformations. We report the first case of a child, as far as we know, with neurofibromatosis type I and subarachnoid hemorrhage caused by rupture of an arteriovenous malformation. CLINICAL CASE: A 9 year old girl presented with the acute onset of a condition preceded by vomiting, deterioration in her level of consciousness and neck rigidity caused by subarachnoid and intraventricular hemorrhage. On angiography a right interpeduncular arteriovenous malformation was seen which arose from the right anterior choroid artery. The patient was admitted to the intensive care unit for stabilization of her condition, followed by surgical operation and good subsequent progress. CONCLUSIONS: We review the cerebrovascular complications associated with neurofibromatosis type I that have been described in the literature. Neurofibromatosis type I may be associated with cerebrovascular complications. The most frequent of these are occlusive or stenotic, isolated or with a vascular pattern of progressive cerebral artery occlusive disease of moyamoya type and aneurysms. Other rarer cerebrovascular complications include fistulas and arteriovenous malformations. The patient we report is the first case of vascular malformation in children associated with neurofibromatosis type I as far as we are aware.

Cerebral Angiography↗
PubMedDate not supplied

[Intradural spinal arachnoid cyst associated with Noonan's syndrome].

INTRODUCTION: Intradural spinal arachnoid cysts are collections of liquid similar to CSF, caused by a disorder of the arachnoid trabeculae. Noonan s syndrome is a condition involving multiple malformations, with autosomal dominant inheritance, variable penetration and expression and a phenotype similar to that of Turner s syndrome. We describe the case of a girl with Noonan s syndrome who presented with progressive scoliosis and signs and symptoms of a disorder of the spine related to an intradural spinal arachnoid cyst. CLINICAL CASE: A four year old girl with no significant previous clinical history was seen for slow growth (in size and weight), progressive scoliosis and urinary sphincter dysfunction. On physical examination there were features of polymalformation compatible with Noonan s syndrome and thoracic scoliosis. In view of this a spinal MR study was done. This showed an intradural arachnoid cyst from segment T4 to T11, with displacement and spinal narrowing which required a cyst-peritoneal shunt. CONCLUSIONS: Intradural spinal arachnoid cysts are collections of liquid produced due to arachnoid trabeculae. Noonan s syndrome is a condition involving multiple malformations with a phenotype which is very similar to that of Turner s syndrome. It is linked to alterations of tissue elastin which favor the appearance of arachnoid cysts. Arachnoid cysts are an uncommon cause of spinal compression and/or progressive scoliosis. Although this association has not been described previously, in the case of a patient with Noonan s syndrome, with clinical features of a spinal disorder and/or progressive scoliosis, the presence of a spinal arachnoid cyst should be considered.

Arachnoid Cysts↗
PubMedDate not supplied

[The return to the ketogenic diet. What role does it play in the treatment of refractory seizures of infancy?].

UNLABELLED: About one third of the patients with epilepsy are not satisfactorily controlled in spite of correct anticonvulsive treatment. Although the ketogenic diet has been used for refractory epilepsy since the 1920s, over the past ten years it has been used much more. OBJECTIVE: To review the effectiveness, tolerance and adverse effects in 12 paediatric patients who have been on this diet for over three months. PATIENTS AND METHODS: We assessed 15 children, of whom only 12 are included in this review ( 5 boys and 7 girls). After initial joint evaluation by the neuropaediatrician and the Clinical Nutrition Unit the diet was started in hospital with a period of fasting (24 48 hours) until ketosis occurred. The diet was continued over three days before the child was sent home. In our centre we used a diet with modified MCT, in which 30% of the energy was given in the form of MCT and 40% as natural fats. The effectiveness of the diet was found by comparing the number of seizures suffered by the patient before starting the diet with the number at different times later (1, 3, 6, 12, 18 months). RESULTS: The median age when starting on the diet was 3 years and 5 months (range 18 months to 9 years). All had been diagnosed as having the Lennox Gastaut syndrome, six having cryptogenic disease. Six children had severe mental retardation. Six months after starting the diet, half the children had over 50% reduction in seizures whilst a third had no seizures at all or had their frequency of occurrence reduced by over 90%. After one year three families had given up the diet, two because of its inefficacy in controlling the seizures. The diet was well tolerated in all cases, with levels of ketonuria maintained at 2+. Mild adverse effects were seen in only three patients and transient rise in the plasma cholesterol level in four children. CONCLUSIONS: The ketogenic diet is still an effective treatment for epilepsy, especially in patients in whom the drugs available have not led to improvement. Its excellent tolerance and few short term side effects encourages its use in most cases of refractory epilepsy.

Anemia, Hypochromic↗
PubMedDate not supplied

[Intracranial arachnoid cysts. A study of a series of 35 cases].

INTRODUCTION: Arachnoid cysts are cavities with a content similar to cerebrospinal fluid, frequently communicating with the subarachnoid space. They make up 1% of the intracranial space occupying lesions, and although typically seen in children (13%), they may be undiagnosed until the patient has become adult. PATIENTS AND METHODS: We review a series of 35 cases of congenital intracranial arachnoid cysts in children, which had been investigated in the Neuropaediatric Department during 1987 1999. The patients had had cranial CAT and MR, and transcranial echography studies. RESULTS: In 85.7% there was a single cyst. The Sylvan fissure and posterior fossa were the commonest sites. The age of diagnosis was under 1 year in 54.3% and at birth in 25.7%. There were other associated malformations of the central nervous system in 31.4%, with agenesis of the corpus callosum in 45.5%. The most frequent clinical features on presentation of the condition were macrocephaly (31.4%) and epileptic crises (25.7%). The surgical treatment most often used was cystoperitoneal shunt. CONCLUSIONS: Congenital arachnoid cysts are caused by alteration in the embryogenesis of the central nervous system, and hence their association with other malformations and prevalence in children. The most frequent clinical findings were macrocephaly, due to the size of the cyst or the associated hydrocephaly, and epileptic seizures secondary to cortical irritation caused by pressure. Management of symptomatic lesions is surgical. The cystoperitoneal shunt was generally used in our series, since this was followed by clinical improvement in most cases.

Adolescent↗
PubMedDate not supplied

[Cerebral cavernomas in childhood. clinical presentation and diagnosis].

INTRODUCTION: Intracranial vascular malformations are congenital lesions due to alterations in the development of the arteriolocapillary network. Traditionally they are divided into four types according to their histological characteristics: arteriovenous malformations, cavernous angiomas or cavernomas, venous angiomas and telangectasias. The cavernomas are multilobulated lesions which are clearly delimited and contain blood at different stages of evolution. Histologically they are composed of sinusoidal spaces lined by endothelium and closely interlinked, without intervening nervous tissue. They are usually found at a supratentorial level, and less frequently in the posterior fossa. PATIENTS AND METHODS: We made a retrospective review of the histories of 11 patients aged between 2 and 10 years, diagnosed as having intracranial cavernomas in which imaging techniques lead to the diagnosis of intracranial cavernoma. In two patients there were multiple cavernomas. We analysed the method of diagnosis and clinical features on presentation. RESULTS: The clinical features were varied. In six patients the onset of the disorder was with epileptic seizures, three had progressive neurological deficits, one patient had headache and vomiting which progressed to coma and another had a history of non specific fainting and deficit. All but one of the cavernomas were supratentorial. CONCLUSIONS: Cavernomas are a type of vascular malformation with specific histological features. The usual clinical features are convulsions and parenchymatous bleeding. They are generally supratentorial. The appearance of MR has permitted diagnosis of asymptomatic cavernomas and is currently considered to be the technique of choice for diagnosis.

Adolescent↗
PubMedDate not supplied

[Unilateral isolated paralysis of the soft palate: a case report and a review of the literature].

INTRODUCTION: Unilateral isolated paralysis of the soft palate is a rare clinical entity. CASE REPORT: We describe the case of a 12 year old girl who presented acute dysphagia, a nasal voice and regurgitation of liquids into the nose. Exploration revealed right velopalatine insufficiency with normal gag reflex and pharyngeal sensitivity. All the complementary studies, including magnetic resonance, lumbar puncture and viral serology tests, were normal. There are 28 similar cases in the literature, with the following characteristics: acute onset, appearing in infancy (96%), predominance in males (79%), recent respiratory infection (35%) and an excellent prognosis for recovery (85%). CONCLUSION: This is probably a case of acute cranial mononeuropathy with a viral aetiology

Adolescent↗